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  • Title: A fluorimetric assay of steroid sulphatase in leukocytes: evidence for two genetically different enzymes with arylsulphatase C activity.
    Author: van Diggelen OP, Konstantinidou AE, Bousema MT, Boer M, Bakx T, Jöbsis AC.
    Journal: J Inherit Metab Dis; 1989; 12(3):273-80. PubMed ID: 2533306.
    Abstract:
    Arylsulphatase C (ASC) activity in leukocytes and fibroblasts measured with 4-methylumbelliferylsulphate, is caused by at least two genetically different sulphatases. One of these is steroid sulphatase (STS). Depending on the substrate concentration, about 10-50% of the ASC activity in leukocytes can be attributed to sulphatases other than STS. Steroid sulphatase can be measured specifically with 4-methylumbelliferylsulphate as the fraction of total ASC activity which is inhibitable by dehydroepiandrosterone sulphate. Using this assay, the adjusted ASC activity in leukocytes from patients with X-linked ichthyosis was 2% of normal. Obligate heterozygotes showed reduced activity.
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